Behchet's Disease

Behchet's Disease
Presentation by Anna Mae Smith, MPAS, PA-C
Lock Haven University

* Rare multisystem, chronic disease characterized by oral and genital mucocutaneous ulcerations, skin rashes, arthritis, thrombophlebitis, uveitis, colitis, and neurologic symptoms.
* Endemic in Japan and Northeastern Mediterranean region(Turkey & Iran)

EPIDEMIOLOGY
SIGNS & SYMPTOMS

o Aphthous stomatitis
o Genital ulcers - painful in the male, painless in the female
o Dermal - papulovesicular, erythema nodosum, pathergy, erythema multiforme, vasculitis, pyoderma
o Ocular - iritis, iridocyclitis, chorioretinitis, hypopyon, hemorrhage, papilledema, optic atrophy
o Morning stiffness - in 1/3
o Polyarthritis - self-limited and predominantly affecting lower extremities
o Thrombophlebitis - peripheral, pulmonary, cerebral, Budd Chiari syndrome
o Neurologic - cranial nerve palsy, hemiplegia, intracranial hypertension, meningomyelitis and recurrent meningitis, confusional state
o Gl - aphthous ulcers, colitis, melena
o Pulmonary infiltrates - possibly related to thrombosis
o Myopathy/myositis - rare
o Peripheral gangrene - rare
o Epididymitis
o Glomerulonephritis - rare

HYPOPYON – pus in the anterior chamber associated with uveitis

Diagnostic criteria

* Recurrent oral ulcers at least 3 times in a year
* Recurrent genital ulcers
* Eye inflammation
* Skin inflammation
o Pseudo folliculitis
o Acne-like lesions
o Erythema nodosum-like

DIFFFERENTIAL

o Reiter's syndrome and other forms of spondyloarthropathy
o Inflammatory bowel disease (Crohn's disease and ulcerative colitis)
o Syphilis
o Erythema nodosum
o Aphthous stomatitis
o Herpes simplex
o Stevens-Johnson syndrome
o Vasculitis
o Multisystem disease
o Thrombophlebitis related to coagulation factor deficiency

LABS

o Erythrocyte sedimentation rate elevation, but can be normal
o Immune complexes detected by Raji cell and C1q solid phase assays
o Cryoglobulin
o Hypergammaglobulinemia
o Circulating anticoagulation (rare)
o Anti-cardiolipin antibody (rare)

Special Tests

* None specific for Behcet's, but helpful in following disease course:
o Depression of plasma antithrombin III levels with active disease
o Increased fibrinolytic activity during attacks
o Anti-neutrophil cytoplasmic antigen antibodies, perinuclear variety
o Demyelinating antibodies in neuro-Behcet's syndrome
o Anti-cardiolipin antibodies, lupus anticoagulants
o Anti-endothelial antibodies


TREATMENT
Complications

o Death
o Blindness
o Paralysis
o Embolism/thrombosis - pulmonary, vena cava, peripheral
o Aneurysms
o Amyloidosis
o Thrombotic events, especially when anticardiolipin antibodies present
o Normal life expectancy, except with neurologic involvement
o Possible vision impairment
o Avoid English Walnuts!!

SWEET SYNDROME

Behchet's Disease.ppt